Bicuspid Aortic Valve Disease

Congenital heart valve defects where the AORTIC VALVE has two instead of normal three cusps. It is often associated with AORTIC REGURGITATION and AORTIC INSUFFICIENCY.

Also Known As:

Aortic Valve Disease 1; Aortic Valve, Bicuspid; Bicuspid Aortic Valve; Bicuspid Aortic Valve, Two Raphe; Bicuspid Aortic Valves; Valve, Bicuspid Aortic; Bicuspid Aortic Valve, Single Raphe; Bicuspid Aortic Valve, Two-Raphe; Double Raphe Bicuspid Aortic Valve; Purely Bicuspid Aortic Valve; Single Raphe Bicuspid Aortic Valve; Two Raphe Bicuspid Aortic Valve; Two-Raphe Bicuspid Aortic Valve

Networked: 722 relevant articles (13 outcomes, 80 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Aortic Valve Stenosis (Aortic Stenosis)
2.	Aortic Coarctation
3.	Aneurysm (Aneurysms)
4.	Turner Syndrome (Turner's Syndrome)
5.	Pathologic Constriction (Stenosis)

Experts

1.	Chen, Mao: 15 articles (11/2022 - 07/2014)
2.	Feng, Yuan: 15 articles (11/2022 - 07/2014)
3.	De Backer, Ole: 15 articles (02/2022 - 01/2016)
4.	Latib, Azeem: 14 articles (06/2022 - 04/2014)
5.	Colombo, Antonio: 13 articles (02/2022 - 04/2014)
6.	Bavaria, Joseph E: 12 articles (07/2020 - 04/2007)
7.	Wei, Xin: 11 articles (11/2022 - 07/2014)
8.	Zhao, Zhen-Gang: 11 articles (01/2022 - 07/2014)
9.	Yoon, Sung-Han: 11 articles (07/2020 - 01/2016)
10.	Girdauskas, Evaldas: 10 articles (10/2022 - 05/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Bicuspid Aortic Valve Disease:

1.	Proteins (Proteins, Gene)FDA Link 01/01/2023 - "Our functional enrichment analysis of the 28 proteins known to have a role in bicuspid aortic valve disease confirmed that this annotation project has led to an improved interpretation of a heart valve genetic dataset. " 01/01/2021 - "Recent studies have highlighted that many individuals with bicuspid aortic valves also have lower levels of a protein known as NOTCH1, which plays a key signalling role for cells. " 08/26/2023 - "We compared abundances of extracellular matrix (ECM) proteins from excised valve tissues of 88 patients with isolated AVD of normal tricuspid (TAV) and congenital bicuspid aortic valves (BAV), quantified more than 1400 proteins per ECM sample by mass spectrometry, and demonstrated that local ECM preserves molecular cues of the pathophysiological processes. " 01/01/2021 - "Importantly, trafficking-defective Piezo1 variants linked to generalized lymphatic dysplasia and bicuspid aortic valve display reduced fully N-glycosylated Piezo1 protein. " 04/16/2019 - "Bicuspid Aortic Valve Alters Aortic Protein Expression Profile in Neonatal Coarctation Patients."
2.	12- (4'- azido- 2'- nitrophenoxy)dodecanoyl- coenzyme AIBA 12/01/2018 - "In Marfan syndrome, bicuspid aortic valve and COA, medial degeneration is more extensive and severe than in another complex CHD. " 09/01/2011 - "All patients had a clinical history of surgery for coarctation of the aorta (CoA), and all but one had a bicuspid aortic valve. " 11/27/2023 - "This study aimed to longitudinally evaluate aortic root dimensions and elasticity in pediatric Turner syndrome (TS) in relation to known cardiac implications such as coarctation of the aorta (CoA) and bicuspid aortic valves (BAV) in order to create an improved risk profile for the presumed underlying vessel pathology in childhood. " 12/07/2022 - "Echocardiography and CT revealed supravalvular mitral membrane, PMV, Bicuspid aortic valve stenosis, CoA and patent ductus arteriosus. " 01/01/2022 - "Coarctation of the aorta (CoA) and bicuspid aortic valve (BAV) often cooccur and are genetically linked congenital heart defects (CHD). "
3.	Matrix Metalloproteinases (MMPs)IBA 01/01/2018 - "The aim of this study was to test whether enlarged size and impaired elastic properties of the ascending aorta are associated with impaired endothelial function and increases in plasma matrix metalloproteinase (MMP)-2 concentrations in patients with bicuspid aortic valve (BAV) without significant valvular dysfunction. " 02/01/2016 - "Bicuspid aortic valve (BAV) exhibits a clinical incline toward aortopathy, in which aberrant tensile and shear stress generated by BAV can induce differential expression of matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs). " 01/01/2016 - "Patients with a bicuspid aortic valve (BAV) are at increased risk for progressive aortic dilation associated with extracellular matrix (ECM) degradation by matrix metalloproteinases (MMP). " 02/01/2012 - "Aortic dilatation with bicuspid aortic valves: cusp fusion correlates to matrix metalloproteinases and inhibitors." 12/01/2004 - "Tissue microarray detection of matrix metalloproteinases, in diseased tricuspid and bicuspid aortic valves with or without pathology of the ascending aorta."
4.	Coenzyme A (CoA)IBA 01/01/2023 - "60-85% of COA patients have a bicuspid aortic valve (BAV). " 12/01/2022 - "Many patients with CoA also have a bicuspid aortic valve (BAV). " 12/09/2021 - "Consequently, she is referred to the cardiologist, who identifies a well-functioning bicuspid aortic valve and a severe CoA with extensive collateral circulation. " 01/01/2021 - "Interestingly, in a subgroup analysis of CoA patients with tricuspid vs. bicuspid aortic valves (BAV), only the latter had increased Aix75. " 12/01/2019 - "CoA was located paraductally in most patients (64%), and bicuspid aortic valve was present in 39 (43%) patients. "
5.	Biomarkers (Surrogate Marker)IBA 09/01/2022 - "To determine the relationship between aortic distensibility and left ventricular (LV) remodeling, myocardial strain and blood biomarkers in patients with stenotic bicuspid aortic valve (BAV) and preserved ejection fraction (EF) by cardiovascular magnetic resonance (CMR). " 01/01/2022 - "Identification of hemodynamic biomarkers for bicuspid aortic valve induced aortic dilation using machine learning." 07/01/2021 - "Plasma proteomic profiling reveals biomarkers associated with aortic dilation in patients with bicuspid aortic valve." 01/01/2020 - "Blood biomarkers in patients with bicuspid aortic valve disease." 01/01/2019 - "Herein we summarize the current knowledge on the bicuspid aortic valve (BAV)-associated aortopathy regarding clinical presentation and disease sub-classification, genetic background, hemodynamics, histopathology, cells and signaling, animal models, and biomarkers. "
6.	ElementsIBA 10/01/2018 - "Investigation of raphe function in the bicuspid aortic valve and its influence on clinical criteria-A patient-specific finite element study." 10/01/2010 - "Biomechanical implications of the congenital bicuspid aortic valve: a finite element study of aortic root function from in vivo data." 01/01/2023 - "The existence of an anomalous bicuspid aortic valve (BAV) is contemplated as a potential etiological element in this context. " 01/01/2022 - "Fully Three-Dimensional Hemodynamic Characterization of Altered Blood Flow in Bicuspid Aortic Valve Patients With Respect to Aortic Dilatation: A Finite Element Approach." 01/01/2020 - "In order to identify a direct link between leaflet stress and calcification development, we performed patient-specific finite-element (FE) analyses of six bicuspid aortic valves (BAV), where the leaflets, raphe and calcifications were all considered. "
7.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 10/01/2011 - "Expression of chondromodulin 1 demonstrated a 6-fold downregulation (P = .0003) and expression of vascular endothelial growth factor demonstrated a 2-fold increase (P = .02) in congenital bicuspid aortic valves compared with that seen in tricuspid valves. " 06/01/2012 - "In our recent study, we first reported that mutation in vascular endothelial growth factor-A is associated with bicuspid aortic valve stenosis. " 07/01/2012 - "Recently, various human studies have revealed a high coincidence of VEGF and NOTCH polymorphisms with cardiovascular outflow tract anomalies, such as bicuspid aortic valves and Tetralogy of Fallot, next to predisposition for cardiovascular pathologies, including atherosclerosis and aortic valve calcification. "
8.	Indicators and Reagents (Reagents)IBA 09/01/1978 - "This proved a more sensitive indicator of a bicuspid aortic valve than the finding of an eccentric aortic valve closure line. " 01/01/2021 - "This study sought to compare aortic stenosis (AS) progression rates, AS-related cardiac damage (AS-CD) indicator incidence and determinants, and survival between patients with tricuspid aortic valve (TAV)-AS and those with bicuspid aortic valve (BAV)-AS. " 06/23/2016 - "In this study, we aimed to evaluate the associations of neutrophil to lymphocyte ratio (NLR; an indicator of inflammation) with left ventricular ejection fraction and ascending aorta diameter in patients with a bicuspid aortic valve (BAV). "
9.	CytokinesIBA 01/01/2019 - "Several histopathologic studies have demonstrated that the development and progression of alterations in bicuspid aortic valve are related to an active process that includes: oxidative stress, shear stress, endothelial dysfunction, disorganized tissue architecture, inflammatory cells and cytokines. " 01/01/2019 - "In this review, we focused on interactions and transitions between aforementioned mechanisms demarcating ascending stages of CAVD, suggesting a predisposing condition (bicuspid aortic valve) and drug combination (lipid-lowering drugs combined with angiotensin II antagonists and cytokine inhibitors) for the further testing in both preclinical and clinical trials."
10.	Transforming Growth Factor-beta Type II ReceptorIBA 04/11/2013 - "The purpose of our study was to investigate the potential contribution of germline mutations in NOTCH1, GATA5 and TGFBR1 and TGFBR2 genes in a cohort of Italian patients with familial Bicuspid Aortic Valve (BAV). " 04/11/2013 - "Sequencing of NOTCH1, GATA5, TGFBR1 and TGFBR2 genes in familial cases of bicuspid aortic valve." 09/01/2008 - "Absence of TGFBR1 and TGFBR2 mutations in patients with bicuspid aortic valve and aortic dilation." 09/01/2008 - "Because bicuspid aortic valve (BAV) is a congenital heart defect in patients with Loeys-Dietz syndrome, this study was conducted to investigate whether variants in TGFBR1 or TGFBR2 are responsible for sporadic BAV. "

Therapies and Procedures

1.	Transcatheter Aortic Valve Replacement 03/01/2015 - "The purpose of this study is to evaluate the safety and efficacy of transcatheter aortic valve implantation (TAVI) in patients with a severe stenotic bicuspid aortic valve (BAV) in a Chinese population. " 07/01/2022 - "Safety and efficacy of transcatheter aortic valve implantation in stenotic bicuspid aortic valve compared to tricuspid aortic valve: a systematic review and meta-analysis." 01/01/2022 - "We sought to conduct a systematic review and meta-analysis of clinical adverse events in patients undergoing transcatheter aortic valve replacement (TAVR) with bicuspid aortic valve (BAV) vs. tricuspid aortic valve (TAV) anatomy and the efficacy of balloon-expandable (BE) vs. self-expanding (SE) valves in the BAV population. " 03/01/2015 - "Evaluation of the safety and efficacy of transcatheter aortic valve implantation in patients with a severe stenotic bicuspid aortic valve in a Chinese population." 01/01/2015 - "We sought to evaluate the safety and efficacy of transcatheter aortic valve implantation (TAVI) in patients with bicuspid aortic valve (BiAV). "
2.	Replantation 06/01/2022 - "Reply: Reimplantation should be the gold standard to treat the regurgitant bicuspid aortic valve." 06/01/2022 - "Reimplantation should be the gold standard to treat the regurgitant bicuspid aortic valve." 12/01/2011 - "In bicuspid aortic valve repair, root replacement with the reimplantation technique stabilizes the ventriculoaortic junction, improves valve mobility (low gradient), and is associated with improved outcomes." 01/01/2022 - "The aim of this anatomical study is to compare aortic reimplantation with Valsalva graft with aortic external ring annuloplasty in bicuspid aortic valves, analyzing their morphological features with CT scan. " 01/01/2019 - "The study consisted of 92 consecutive adult patients (tricuspid aortic valve group = 63 and bicuspid aortic valve group = 29) who underwent aortic valve sparing aortic root reimplantation surgery with or without aortic cusp repair for dilatation of the aortic root and/or aortic valve regurgitation at our institution from April 2004 to October 2016. "
3.	Therapeutics 01/01/2016 - "No medical therapy has been proven to prevent the progression of aortic dilatation in bicuspid aortic valve (BAV) disease, and prophylactic aortic surgery remains the mainstay of treatment. " 11/08/2021 - "Advances in Transcatheter Aortic Valve Therapy in Bicuspid Aortic Valve Disease: Insight into Patient Selection, Management, and Future Directions." 12/01/2020 - "Commentary: Can we move beyond aortic size, using real-time analysis of aortic tissue, to more precisely guide therapy for patients with bicuspid aortic valves?" 01/01/2019 - "Standard therapy for aortic root dilation in the setting of bicuspid aortic valves remains use of a composite valve conduit. " 04/01/2016 - "Influence of beta-blocker therapy on aortic blood flow in patients with bicuspid aortic valve."
4.	Prostheses and Implants (Prosthesis) 12/01/2022 - "In the context of SAVR with RAT, Perceval has been considered the "perfect marriage." In patients with bicuspid aortic valve (BAV), some surgeons initially avoided the Perceval valve but, with growing experience, the prosthesis has been used for a wide variety of indications. " 12/01/2022 - "Outcomes after aortic valve replacement in children with bicuspid aortic valve are excellent in the short and long term, regardless of using mechanical or biological prostheses." 10/01/2022 - "The role of sutureless or rapid-deployment aortic valve prosthesis in bicuspid aortic valve: Standard AVR, TAVR, or somewhere in between?" 10/01/2022 - "The objective of this scoping review is to describe the postoperative outcomes and complications of patients with bicuspid aortic valve (BAV) treated with sutureless or rapid-deployment prosthesis. " 10/01/2022 - "The use of sutureless and rapid-deployment aortic valve prosthesis in patients with bicuspid aortic valve: A focused review."
5.	Contraindications 01/01/2023 - "Bicuspid aortic valve (BAV) is also no longer considered a contraindication to TAVI; however, the effect of BAV on postoperative conduction disturbances after TAVI is unknown. " 11/16/2022 - "Bicuspid aortic valve and aortopathy are generally considered contraindications to isometric exercise. " 07/01/2016 - "Bicuspid aortic valve (BAV) is generally considered to be a contraindication to sutureless aortic valve replacement (AVR). " 10/01/2015 - "Bicuspid aortic valve (BAV) is generally considered to be a contraindication to sutureless aortic valve replacement (AVR). " 02/01/2015 - "Historically, the presence of a bicuspid aortic valve (BAV) has been regarded as a contraindication to TAVI, on the basis of putative concerns about the associated risks of elliptical deployment, accelerated leaflet degeneration, periprosthetic leaks, and aortic complications. "